These resources include the best available evidence about the course of Rett syndrome and its management as well as practical information about family associations, specialist clinical centres and links to relevant websites.
Marr, C., Leonard, H., Torode, I., & Downs, J. (2015). Spinal fusion in girls with Rett syndrome: Family experiences and postoperative recovery. Child: Care, Health & Development. Download snapshot, see article abstract.
Scoliosis occurs commonly in Rett syndrome. The surgical procedure of spinal fusion may be recommended to families if their daughter's scoliosis is progressive. This study investigated recovery following spinal fusion of girls with Rett syndrome during the first 12 postoperative months. Family perspectives and coping around the time of surgery are also explored.
What we did
Families of girls who underwent a spinal fusion between 2006 and 2012 were recruited from the Australian Rett Syndrome Database. Twenty-five parent interviews were conducted to explore the course of their daughter's recovery following discharge from hospital. Parents also discussed stress and coping with their daughter's spinal fusion surgery.
What we found
Some of the girls experienced poor appetite and constipation in hospital, which improved after they were discharged. The majority of girls recovered their preoperative sitting, standing and walking ability within 12 months of undergoing the procedure, and some developed stronger endurance when doing these activities. Postoperative complications and other health issues (e.g., a later bone fracture) influenced a very small portion of girls whose recovery took longer.
Parents' decision to proceed with surgery was associated with mixed feelings, likely due to the complexity of the procedure and concerns as to how their daughter would recover. We found that parents needed a comprehensive range of supports, including information and discharge preparation, confidence in themselves and hospital staff, and the ability to balance their need for personal time with their daughter's care following the surgery. These support strategies will help reduce parents' emotional burden and manage their daughter's and family's health following discharge from hospital.
What it means
This study identified clinical practice issues in relation to recovery following spinal fusion. For most, return of wellness and preoperative gross motor skills following spinal fusion occurred within the first 12 postoperative months. Parents require information to prepare for discharge as well as practical supports.
For further reports and findings, see our publications list.